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Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants.

Ten infants with classical maple syrup urine disease (MSUD) and two with variant MSUD had a total of 26 CT scans and 13 MR examinations of the brain during different stages of their disease. We found that inter- and intrapatient analyses of CT and MR findings at times ranging from 3 days to 7 months were typical enough to suggest the MSUD diagnosis. Imaging studies showed the natural course of the disease and, in a few cases, illustrated the effects of therapy. CT scans typically are negative during the first few days of life, then a marked, generalized, diffuse edema appears. In addition, a localized, more severe edema (the MSUD edema) is seen, which involves the deep cerebellar white matter, the dorsal part of the brainstem, the cerebral peduncles, and the dorsal limb of the internal capsule. Both the generalized and the MSUD edema subside during the second month of life, then may disappear totally or leave a well-defined, low-density zone around the lateral ventricles and small, low-attenuation lesions within the brainstem, respectively. With the disappearance of the edema, some loss of brain substance becomes obvious.

Brain↗

[Stereotactic aspiration using coordinate software for hypertensive intracerebral hematomas].

Stereotactic aspiration for intracerebral hematoma has become widely used due to improvements in computed tomography (CT). CT-guided stereotactic aspiration for hypertensive intracranial hematomas was performed in 46 cases. Stereotactic aspiration with coordinate software was carried out among 16 cases. We compared the clinical results of conventional stereotactic aspiration with results of the new method using coordinate software. Using coordinate software, we can examine the route for the target point, as well as the target point of the hematoma before surgery. Therefore we can avoid obstacles such as ventricles and internal capsules. Further, we can aspirate hematoma by using only this one procedure. Mean hematoma aspiration percentage has risen from 44.9 to 87.2% by using coordinate software. Rate of drainage tube insertion after operation has decreased from 90 to 50%. The period of placement of the drainage tube and the frequency of infection due to the drainage tube has become lower, too. Therefore we can say that the outcome of treatment of hypertensive intracranial hematomas is improved by using coordinate software.

Cerebral Hemorrhage↗

[Three cases of cryptic arteriovenous malformation in basal ganglia manifested by putaminal hemorrhage].

Three cases of histologically proven cryptic arteriovenous (AV) malformation of the basal ganglia are reported. Seventeen cases of patients showing putaminal hemorrhage in the CT scan underwent craniotomy for evacuation of a hematoma between January 1986 and December 1988. The preoperative diagnosis was hypertensive putaminal hemorrhage. In all three cases a network of abnormal vessels around the internal capsule was detected at surgery. Case 1: A 62-year-old male was admitted to our hospital with consciousness disturbance (JCS 20), total aphasia and right hemiparesis. The CT scan showed left putaminal hemorrhage. The volume of the hematoma was 45 ml by CT criteria. Left carotid angiography did not demonstrate any abnormal findings. Case 2: 69-year-old male was admitted with consciousness disturbance (JCS 10) and right hemiparesis. Left putaminal hemorrhage (volume 25 ml) was recognized on the CT scan. Two hours after admission, the consciousness level suddenly deteriorated (JCS 100). At that moment, the volume of the putaminal hematoma had increased to 100 ml. AV malformation was not detected by left carotid angiography. Case 3: A 50-year-old male was admitted with consciousness disturbance (JCS 20), total aphasia and right hemiparesis. The CT scan showed left putaminal hemorrhage of 73 ml. Pathohistological examination proved AV malformation in each case. The three cases described here suggest the following; 1) The incidence of the basal ganglia cryptic AV malformation has been considered low, but meticulous examination shows that among the cases diagnosed as hypertensive hemorrhage, AV malformation is not infrequently the hidden cause.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

MRI of intraparenchymal hematoma: responsible mechanisms.

The MRI appearance and evolution of hemorrhage remains a difficult entity. A typical example of the MR visualization of an intracerebral bleeding in the basal ganglia and the internal capsule at 1.5 T is presented. The sequential biochemical and physical changes in an hemorrhage are described.

Adult↗

[Diagnostic value of short latency somatosensory evoked potentials for various intracranial lesions].

Diagnostic value of short latency somatosensory evoked potentials (SSEP) was studied in 124 patients with various intracranial lesions. Abnormal SSEPs were recorded in 58 of 124 patients (46.8%) and were classified into three types. Type I (6 cases) showed abnormality of late components with N18 being preserved. All patients with type I abnormality had cortical or subcortical lesions in the parietal lobe. Type II (20 cases) was characterized by abnormality of N18 and late components with N16 being preserved, and mainly seen in patients with a lesion involving thalamus and internal capsule. Type III (31 cases) showed abnormality of N16 and N18 which was elicited by unilateral stimulation (IIIa: 11 cases), or bilateral stimulation (IIIb: 21 cases), and this indicated brainstem impairment. The incidence of SSEP abnormality was as high as 93.1% in patients with sensory disturbance, and 23.1% in patients without such disturbance, and it was suggested that SSEP is useful to detect subclinical dysfunction in the somatosensory pathway. The SSEP grades defined by Anderson et al were found to be well correlated with the outcome of patients with severe head injury, and the SSEP was more reliable for predicting the outcome of patients than the auditory brainstem evoked responses. The SSEP grades were also fairly well correlated with the outcome of patients with cerebrovascular accidents, although the outcome was not consistent in patients with moderately abnormal SSEP.

Adolescent↗

[Hemiplegia: diagnosis and differential diagnosis].

Hemiplegia is the most frequent form of paralysis in humans and involves face, arm and leg on one side of the body. Diseases localized in the cortex, the cerebral white matter (corona radiata) and the internal capsule usually manifest themselves by weakness or paralysis of the face, the arm and the leg on the opposite side. In the causation of hemiplegia, vascular diseases of cerebrum and brainstem exceed all others in frequency. Trauma ranks second, and other important causes are brain tumor, encephalitis or abscess and demyelinating diseases.

Central Nervous System Diseases↗

[Cerebellar ataxia and unilateral asterixis caused by thalamic hematoma. Presumed mechanisms].

A 76 year-old hypertensive man developed an acute inability to stand due to a right cerebellar ataxia. Somatosensory performances were normal, but a transient and mild weakness of the right arm and leg with Babinski's sign was observed. There was a prominent asterixis of the right hand. CT scan showed a hemorrhage of the thalamus with surrounding edema of the adjacent internal capsule. Initial median nerve somatosensory evoked potentials showed a mild reduction of left parietal responses with absent left frontal SEPs (P22 and N30). Fourty days later the cerebellar ataxia was persisting while asterixis had disappeared. A second recording of SEPs showed a complete recovery of all cortical components. MRI performed at the same time showed a left postero lateral thalamic lesion. CT, MRI and SEPs findings suggested that asterixis could result from interruption of somatosensory fibres projecting to the motor cortex.

Aged↗

Generation and characterization of monoclonal antibodies against rat microglia and ontogenic distribution of positive cells.

We have generated two mouse IgM monoclonal antibodies against rat microglia. The RMG-1 antibody recognized a 46-kilodalton cell-membrane antigen of ameboid microglia and cross-reacted with perivascular cells, monocytes/macrophages, and capillary endothelial cells of various organs but not with ramified microglia. In the normal brain, RMG-1-positive cells were found in the choroid plexus, periventricular area, corpus callosum, subarachnoid space and white matter of the cerebrum. The RMG-2 antibody recognized a 78-kilodalton cytoplasmic and cell-membrane antigen of ameboid and ramified microglia and cross-reacted with perivascular cells and monocytes/macrophages of various organs but not with endothelial cells. A small number of RMG-2-positive cells were found in the choroid plexus and subarachnoid space on embryonic day 17. On postnatal day 1, RMG-2-positive cells appeared in the periventricular area and corpus callosum and then migrated to the internal capsule and thalamic nucleus. Thereafter, the number of RMG-2-positive cells increased, reaching a maximum on postnatal day 10 to 14, and gradually decreasing by postnatal day 28. These observations show that microglia are cells of monocytic lineage that enter the brain parenchyma late in embryogenesis or early in the postnatal period and lose at least some monocytic antigens to differentiate into ramified microglia. These monoclonal antibodies will provide useful tools to investigate the relation between microglia, perivascular cells, and endothelial cells in the brain and the kinetics of these cells in normal or pathologic conditions.

Aging↗

[Parkinsonism associated with cerebrotendinous xanthomatosis].

Two sibling cases of cerebrotendinous xanthomatosis with parkinsonism were reported. One was a woman of 39 years old, and another was her sister of 36 years old. In both cases, febrile convulsion appeared on 1.5 year old, and mental deterioration, ataxic -spastic gait, cataract and swelling of Achilles tendons developed in order since entrance into elementary school. Five years ago, while they were in hospital at the first time, they were diagnosed as cerebrotendinous xanthomatosis by mental disturbance, cerebellar ataxia, pyramidal tract sign, histologically xanthomatous granuloma of Achilles tendons and hypercholestanolemia and family history of autosomal recessive trait. After the second admission, parkinsonism was noticed in addition to those findings above. Parkinsonism consisted of the following: Resting tremor of parkinsonian type, mild muscle rigidity of forearm and intrinsic-plus hand were observed in the elder sister, and generalized severe rigidity and bradykinesia in the younger sister. In both cases, brain CT showed the pontocerebellar atrophy, and the bilateral low density area in corona radiata, posterior portion of internal capsule, cerebral peduncle, tegmentum of midbrain and deep matter of cerebellum. Brain MRI also showed abnormal intensity in the same regions as on the brain CT. Administration of anti-parkinsonian drugs was challenged for the parkinsonism. Oral L-dopa test (500 mg) moderately improved parkinsonism in both cases. Therapy of diphenylpyraline hydrochloride (10 mg/day) entirely inhibited parkinsonian tremor and mild rigidity in the elder sister but was less effective for severe rigidity in the younger sister than administration of L-dopa.(ABSTRACT TRUNCATED AT 250 WORDS)

Achilles Tendon↗

"Moustache" appearance in craniopharyngiomas: unique magnetic resonance imaging and computed tomographic findings of perifocal edema.

This report describes two cases of craniopharyngioma with perifocal edema. In both patients, computed tomography and magnetic resonance imaging (MRI) revealed that the tumors occupied the suprasellar cistern, invaginated the floor of the 3rd ventricle and were tightly adherent to the ventricular walls. The intraventricular portions of the tumors were cystic, containing protein-rich fluid as suggested by MRI and confirmed by operative findings. There was perifocal edema in the hypothalamus adjacent to the intraventricular tumor, the optic tracts, and the posterior limbs of the internal capsules, resembling the shape of a moustache on axial computed tomographic and MRI scans. The perifocal edema subsided after treatment of the intraventricular tumor by surgical resection or radiation therapy. The "moustache" appearance seems a unique, characteristic feature of perifocal edema, which is observed infrequently with certain craniopharyngiomas.

Adult↗

Quantitative assessment of somatic muscle tone.

An operational definition and a method for the evaluation of somatic muscle tone are presented. The relation between the applied perturbation (pseudo-random sequence of torque pulses) and the resulting changes in joint angular position defines a mechanical admittance. The usefulness of this and related concepts for the quantitative assessment of pathologic changes in muscle tone is illustrated with an example of an experimental lesion of the internal capsule in the monkey. The identification of time-varying reflex responses is discussed in relation to a catching task in human subjects.

Electromyography↗

[A case of hemiballismus during cryptococcal meningitis].

We present a case of hemiballismus related to cryptococcal meningitis. A 23 year-old man was hospitalized because of involuntary movements of his left side, confusion, hyperpyrexia, neck stiffness, bilateral papilledema, right hemiparesis and bilateral pyramidal signs. Diagnosis was made by CSF examination demonstrating cryptococci by india ink. CT with contrast showed hyperdense lesions in the head of the right caudate nucleus, in the left internal capsule and in the frontal and occipital lobes. After treatment with amphotericin B, 5-fluorocytosine and haloperidol, he experienced rapid recovery with disappearance of hemiballismus. To our knowledge, this is the first report of hemiballismus caused by cryptococcal meningitis.

Adult↗

[An experimental study of acute brain ischemia followed by reperfusion in rhesus monkeys].

Acute brain ischemia was accomplished in 18 rhesus monkeys through bilateral occlusion of the vertebral and carotid arteries. The temporary blood volumes (TBV) of the corresponding areas of the ischemic brains were compared with those of the normal controls. At the time of brain ischemia the TBV in basilar ganglion and internal capsule decreased significantly (7.28%) and that in the temporal cortex decreased moderately (22.76%) while the TBV in the brain stem did not show significant changes. The amplitude of EEG was suppressed or became isoelectric immediately after 15 minutes of ischemia. The EEG slightly restored following a reperfusion of 120 minutes, but it became irreversible if ischemia lasted more than 30 minutes. The sodium and water content increased as ischemic was prolonged. This was more significant after recirculation. Ultrastructural findings showed that mitochondria, endoplasmic reticulum and astrocytic processes became swollen after 15 minutes of ischemia. When ischemia lasted more than 30 minutes, mitochondria became swollen more significantly with ruptured cresta. There were marked margination of nuclear clumping chromatins. According to these changes the neuron underwent irreversible injury. As ischemia lasted more than 60 minutes these changes became more apparent, i,e, the mitochondria contained amorphous matrix densities. The nuclear chromatin showed aggregated clumping. The cellular membranes were damaged and the blood vessels contained microemboli. After reperfusion it became much more serious.

Animals↗

[Capsular pseudobulbar mutism in a patient of lacunar state].

A patient of mutism with pseudobulbar palsy and frontal lobe syndrome resulting from lacunar state was reported. The patient, a 64-year-old man, was admitted to Gifu University Hospital because of a decrease in spontaneous activity, lack of volition and anorexia. The CT scan, performed on July 29, 1987, demonstrated lacune in the right internal capsule (IC), periventricular lucency especially around the anterior horn of lateral ventricles, and ventricular dilatation. He was transferred to a medical ward because of repeated aspiration pneumonia. Neurological examination revealed mutism, pseudobulbar palsy, and frontal lobe signs. The CT scan, performed on March 30 1988, demonstrated the newly developed lacune in the left IC. The MRI also showed two coinciding lacunes, one in the genu of the right IC and the other in the anterior limb of the left IC. The SPECT with 123I iodoamphetamine showed decreased blood supply predominantly to the frontal lobes. A mechanism by which the mutism occurs is discussed from two points of view, pseudobulbar palsy and frontal lobe syndrome. He developed initially frontal lobe syndrome in which paucity of spontaneous speech was noted. The CT scan at that time demonstrated lacune in the right IC. About eight months later when he became mute, the CT scan showed lacunes in bilateral ICs without any other low density areas in frontal language areas such as Broca's area, subcortical area and supplementary motor area. As the MRI showed that the right lacune was in the genu but the left lacune was in the anterior limb of IC, the left cortico-bulbar tract was thought to be not directly involved. The SPECT showed decreased blood supply predominantly to the frontal lobes. Although dysphagia improved, mutism did not improve at all. Therefore it is postulated that both pseudobulbar palsy and frontal lobe dysfunction might play a role in producing the mutism of this patient.

Akinetic Mutism↗

Brain perfusion SPECT using an annular single crystal camera: initial clinical experience.

The annular single-crystal brain camera (ASPECT) is a digital SPECT system with a single-crystal sodium iodide thallium Nal(Tl) ring detector and collimator system designed to view the patient's head from three angles simultaneously. The ring is rotated concentrically to the detector for three-dimensional reconstruction over a 21.4 cm (diameter) by 10.7 cm (length) field of view. We evaluated the system clinically by imaging a Hoffman brain phantom and seven subjects, of whom two were normal controls, three had previous cerebral infarction and two had dementia. The ASPECT system produced tomographic images of high spatial resolution. In normal subjects, the separation of striata from thalami by the posterior limbs of the internal capsules was much clearer on ASPECT images than on rotating gamma camera images. The high spatial resolution obtained with the ASPECT system translates into superior anatomical representation of the brain compared to the standard rotating gamma camera.

Adult↗

Quantitative analysis of experimental peritumoral edema in cats.

Cat brain tumors were produced by stereotactical xenotransplantation of rat glioma clone F98 into the internal capsule of the left hemisphere. Two to four weeks after implantation, the tissue content of water, sodium, potassium, calcium, magnesium, serum albumin, serum immunoglobulin, and hemoglobin was measured in samples taken from the tumor, from peritumoral white and gray matter, and from homotopic regions of the opposite hemisphere. Extravasated serum protein content was determined by subtracting intravascular from total tissue protein, using the hemoglobin content as a marker of blood volume. The development of brain tumors was accompanied by severe vasogenic brain edema, which was clearly confined to the ipsilateral white matter. The increase of water was paralleled by an increase of sodium, calcium, and serum proteins. Potassium and magnesium content remained constant. The calculated sodium and calcium content of edema fluid approximated that of blood serum. The content of blood proteins was about 50% lower, but the ratio of albumin/immunoglobulin was the same as in blood. It is concluded that peritumoral edema is a combination of plasma ultrafiltrate and whole plasma extravasation with different modes of formation. Implications for the pathophysiology and therapy of peritumoral edema are discussed.

Animals↗

Imaging of water distribution in the rat brain by activation autoradiography.

Regional water distribution in the rat brain was obtained autoradiographically by activation analysis. The autoradiogram obtained for the normal rat brain showed high accumulation of water in the areas of sensory-motor cortex, hippocampus, thalamus, and amygdaloid cortex, whereas corpus callosum and internal capsule showed low water contents as expected. The estimated values of water content were 78.6 +/- 4.9 weight % for gray matter, and 73.5 +/- 4.9 weight % for white matter, respectively. The mean values of the water content were consistent with those obtained by a conventional drying-weighing method.

Animals↗

MR assessment of myelination in infants and children: usefulness of marker sites.

A retrospective study was made of 60 patients, 1 month to 3 years old, to determine the normal progression of white matter myelination on MR imaging. All examinations were performed with a 1.5-T unit, and axial T1- and T2-weighted images were evaluated in each patient. Multiple sites in the cerebral hemisphere and cerebellum were examined in each case for the presence and degree of myelination. The results show that MR imaging is sensitive to the early changes of white matter myelination, and imaging patterns correlate with known patterns from pathologic studies. At the time of birth in a full-term infant the posterior limb of the internal capsule, central corona radiata, and cerebellar peduncles show visible myelination. Myelination in the centrum semiovale then proceeds anteriorly and posteriorly. Both T1- and T2-weighted images show these changes, which are best explained by a decrease in the water content of white matter as myelination progresses. Knowledge of these normal myelination patterns is essential in evaluating MR imaging studies in infants and children and in diagnosing delayed myelination.

Aging↗